Discoid
lupus erythematosus is a rare disease in
children.
There
are fewer than 20 cases reported in the medical
literature.
Studies
found that only 2% of discoid lupus erythematosus
patients have onset of their disease in
childhood, with the youngest patient reported
presenting at one week of age.
A
survey of 204 patients found that only 3
developed discoid lupus erythematosus before age
10.
As
in the adult form of discoid lupus erythematosus,
there are multiple clinical manifestations of
childhood discoid lupus erythematosus.
The
classic lesion is the discoid plaque with
atrophy, follicular plugs, scale, and scarring
alopecia.
In
childhood discoid lupus erythematosus, a family
history of discoid or systemic lupus
erythematosus occurs in 25% of reported cases.
Unlike
the adult form, childhood discoid lupus
erythematosus does not demonstrate a female
preponderance.
Photoexacerbation
occurs in 31% of children with discoid lupus
erythematosus, whereas sun exposure aggravates
the disease in 60% of adults with discoid lupus
erythematosus.
Over
30% of patients with childhood discoid lupus
erythematosus develop systemic symptoms.
There
are no reliable ways to predict which children
will develop systemic disease: equal numbers of
ANA-positive and ANA-negative patients progress
to systemic disease.
Also,
equal numbers of patients with localized versus
disseminated lesions may develop systemic
disease.
The
treatment of childhood discoid lupus
erythematosus consists primarily of controlling
disease activity and preventing scars.
Sun
protection and sun avoidance are the right stays
of prevention, while potent topical
glucocorticoids are used to treat active lesions.
In
refractory cases, hydroxychloroquine, 4 to 6
mg/kg/day, has been helpful.
Close
follow-up is mandatory to watch for signs of
progression to systemic disease.